Clinical Picture of Endemic Cretinism in Central Apennines (Montefeltro)
Donati L; Venturi S; Andreani M; Antonelli A; Gori E; Bertoni F; Pruneti CA; Berrettini S; Bruschini P; Baschieri L
Università di Pisa, Pisa, Tuscany, Italy
Thyroid. 10/1992; 2(4):283-290. DOI: 10.1089/thy.1992.2.283
ABSTRACT. Endemic cretinism is still present in an endemic goiter area of the central Apennines (Montefeltro) (goiter prevalence 55%; mean urinary iodine level 39 mug/g creatinine). Clinical and biochemical features of patients with myxedematous, neurologic, and mixed cretinism were studied. Also, in this area, as in most other, neurologic cretinism is more prevalent than myxedematous and mixed forms.
The hormonal profiles of the three types of cretinism were clearly different. Nevertheless, all myxedematous cretins had some neurologic disorders (hyperreflexia, increased muscle tone, disorder of gait, Babinski sign, hypoacusia) that were similar to those present in neurologic cretins. These findings suggest that neurologic damage is very similar in all forms of endemic cretinism, reflecting a diffuse insult to the developing fetal nervous system.
Furthermore, these data support the hypothesis that the primary pathophysiologic event in the different types of endemic cretinism is represented by maternal and fetal hypothyroidism, while differences may be explained by the extent and duration of postnatal hypothyroidism. All the cretins were over 35 of age, suggesting a severe iodine deficiency in the past decades, and a progressive improvement of nutritional status resulted in ''silent iodine prophylaxis.''
However, recent studies have revealed the persistence of a moderate iodine deficiency, a high prevalence of neurologic hypoacusia, and reduction of mental performance in normal schoolchildren of this area. These findings constitute strong evidence in favor of adequate iodine prophylaxis.
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Iodine Deficiency in the Population of Montefeltro, A Territory in Central Italy Inside the Regions of Emilia-Romagna, Tuscany and Marche
Venturi S.
International Journal of Anthropology Vol. 29 - n. 1-2 (1-12) - 2014
Introduction. Goiter (the abnormal enlargement of the thyroid gland) is the most specific clinical marker of iodine deficiency in the human body. According to WHO, iodine deficiency is the most common cause of preventable brain damage, causing impaired cognitive development in children, and in many countries it is still a major health problem. During pregnancy, severe iodine deficiency may impair fetal development, resulting in cretinism, with irreversible mental retardation and developmental abnormalities, as well as miscarriage and stillbirth.
This is a review of the endocrinological and neuropsychological studies conducted on the iodine deficient population of Montefeltro, a territory in central Italy, where the use of iodized table salt has only recently eradicated endemic malnutrition in its young population. However, in adults and the elderly scars still remain with some neuropsychiatric disorders and the last surviving cases of cretinism. I would also like to point out that research into the biochemical compounds of iodine is a new area of investigation and one which may provide insights into apoptosis, carcinogenesis and many cardiovascular and degenerative diseases.
Key words: iodine deficiency, iodolipids, goiter, cretinism, brain, evolution
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